RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Engelhardt, Thomas
SR Print(0)
ID 1164076517
T1 Leigh Syndrome
T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e
YR 2019
FD 2019
PB McGraw-Hill Education
PP New York, NY
SN 9781259861789
LK accessanesthesiology.mhmedical.com/content.aspx?aid=1164076517
RD 2024/10/14
AB It  is  a  severe  progressive  mitochondrial  disorder  impeding  oxidative  phosphorylation  with  leads  to  necrotizing  encephalopathy  between  the  age  of  3  months  and  2  years.  It  has  also  been  reported  that  the  onset  begins  later  during  childhood.  It  causes  brain,  spinal  cord,  and  optic  nerves  degeneration  and  clinically  is  characterized  by  acquired  motor  skills,  muscle  weakness  and  hypotonia,  lactic  acidosis  associated  with  respiratory  and  kidney  dysfunctions.  The  association  with  hypertrophic  cardiomyopathy  has  been  reported  and  represents  a  major  anesthesia  consideration.  Although  malignant  hyperthermia  (MH)  has  not  been  reported  with  Leigh  disease,  it  may  be  wise  to  avoid  triggering  agents  as  other  myopathic  conditions  with  a  propensity  for  MH  have  been  misdiagnosed  in  the  past  in  the  presence  of  muscular  disorders.